Gabby's Medical Journey Part 1

Gabby's heart defect, tetralogy of Fallot, was diagnosed during a "routine" 20-week prenatal ultrasound. My chances of having a baby with a genetic defect went from 1 in 84 to 1 in 3 instantly. We had the amniocentesis done a few days later, and we found out that other than the heart issue, everything looked fine. By the time she was born, I'd had a total of 8 ultrasounds, including at least 3 by her pediatric cardiologist. The funniest one was when he had to get her to move to get a better look at her heart. As he was re-scanning my belly to look at her heart, he started to laugh. I asked what was so funny, and he answered, "She just shook her fist at me!" That's my Gabby.

For those who don't know, here's a brief explanation of tetralogy of Fallot (abbreviated ToF). Tetralogy means 4 things. In this case, it's an aorta that goes into both the left and right ventricles (it's supposed to only go into the left one), a narrowed outflow tract in the right ventricle, which goes to the lungs (resulting in reduced flow to the lungs), an opening between the 2 ventricles, and overgrowth of the muscle in the right ventricle because it's working so hard to pump blood through the narrowed outflow tract. In Gabby's case, she also had an opening between the 2 atria, but it was kind of minor compared to everything else. What all of this causes is circulation of blood that is not oxygen rich. Not enough blood is getting to the lungs, and if the baby cries (and at other times), that outflow tract narrows even more, causing the oxygen saturation to drop even more since the body is circulating deoxygenated blood. At least blood is able to circulate thanks to the opening between the ventricles. There is a lot of shunting that goes on, which means that blood is forced from the right side of the heart to the left side. All of this results in what are called "tet spells" where the baby looks more blue than before or even loses consciousness due to lower oxygen levels in the blood. You have to try to not let the baby cry much, which can be challenging. This is the defect that a lot of "blue babies" have. They're the babies that look fine when they're first born, then a week or 2 later, they start looking blue. Wikipedia has a pretty good explanation, complete with illustrations. I'm just glad that she was diagnosed before birth so we were prepared.

Gabby was fine when she was first born. She had to be on bilirubin lights for jaundice for several days, just like her sister. She looked great at her first heart check-up. Then she started having tet spells. They were kind of scary. Diaper changes were challenging. We took her back to the clinic, and they started her on medication. During the first visit where her saturations dropped, I saw them drop into the 50's then come back up. The medication didn't help much. She was hospitalized the first time just before she turned 1 month old. She had her first heart cath during that stay. They found a narrowing in the left pulmonary artery, which goes to the left lung. They took a rotor blade to it and expanded it with a balloon in the hopes that it would last her until she was 4 months old, the goal age for her corrective surgery. It was not to be.

I've been known to say that the cath procedure gave us a less-worried Christmas. On New Year's Day, her 2-month birthday, she had her most serious tet spell to date. She woke up from a nap in the early afternoon crying. She was in her carseat in the stroller at the end of the table. We had just finished having lunch. (We had also found out that she had a problem with reflux. This did NOT help matters.) Anyway, Jack picked her up and started flying her through the air in an attempt to get her to calm down. Then she went kind of gray. Jack said, "I don't know if she's asleep or unconscious." I was immediately on the phone to get a hold of the cardiologist on call. I had Jack flick the bottoms of her feet to see how unresponsive she was. It made her cry. That was better to hear than nothing at that point. When the cardiologist heard her cry, she had me calm her down (breastfeeding rocks for this) & transport her to Children's since we were only 20 minutes away and she was more conscious than she'd been. I came very close to calling 911 that day, though.

When we arrived at the ER less than an hour later, I was amazed at how quickly we were ushered in. They were expecting us and knew that she needed immediate attention. As soon as we got to an ER room, they had her hooked up and monitored. She was fine for awhile. But they still wanted to admit her for observation at that time, but they weren't sure if it would be the floor or the ICU. We also had Sarah to care for, and she wasn't quite 2 yet. Keeping her occupied wasn't easy, so Jack took her home since we didn't have a ready babysitter to call at the time. As I was sitting there in the ER with Gabby later, I saw her oxygen sats start to drop again. I quit looking when it hit about 40. All I could think to do was pick her up and hold her with the thought that if she was dying, at least she'd die in my arms. (The cardiologist said that the lowest she saw was 38.) This was not good. She bought herself a bed in the ICU that evening.

One of the options that the cardiologist said we had was to maybe sedate her and put her on a ventilator so they could better regulate her oxygen levels. But it was an option, not a treatment plan just yet. So, when we got to the ICU just after 8 that evening, I noticed a bunch of medical personnel in scrubs gathered at the door to the ICU. Being a nurse, I knew that something was up. I asked, "What's going on?" The doctor said, "We're going to intubate her." At that point, I put my hands up and said, "Wait. I was told that this was a possibility, not definite. I haven't signed a consent for this. I need to talk to my husband." Now, I wasn't being foolish here. I was watching the monitor and saw that her saturations were in the 80's and had been there for awhile. I wanted to be sure that this was really necessary. They were willing to wait, so it was obviously not an emergency. I called Jack. He wanted to know what her regular cardiologist, Dr. Lewin, thought of this. The guys in the ICU got him on the phone for me which really surprised me. Dr. Lewin said that he'd trust this doc with his child's life, and if he said this was what needed to be done, then it probably was. So, I told them that it was ok to intubate her and put her on a ventilator, but I wanted to nurse her first. The doc then asked, "When did she eat last?" I replied, "About 8:00 in the ER." He told me that she couldn't eat for 4 hours beforehand, which was even more proof that this wasn't an emergency. So, we waited until midnight when the same bunch of folks in green scrubs gathered at her door. I looked at her monitor, and it was at 88, which is where it had been since we'd been admitted. I asked the docs, "Her sats have been in the 80's since we got here. Is this really necessary?" I was then told that the fellow was checking with the attending for that very reason. She wound up not having a tube down her throat until her surgery 4 days letter. The lesson here is to always advocate for yourself or your child if you don't understand why something is being done.

To be continued